PrPSc Proteins

Abnormal isoform of prion proteins (PRIONS) resulting from a posttranslational modification of the cellular prion protein (PRPC PROTEINS). PrPSc are disease-specific proteins seen in certain human and animal neurodegenerative diseases (PRION DISEASES).

Also Known As:

Networked: 403 relevant articles (5 outcomes, 44 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Caughey, Byron: 17 articles (08/2014 - 03/2002)
2.	Baron, Thierry: 13 articles (01/2014 - 01/2004)
3.	Bencsik, Anna: 11 articles (02/2012 - 04/2004)
4.	Baron, Gerald S: 8 articles (02/2014 - 03/2002)
5.	Biacabe, Anne-Gaëlle: 7 articles (01/2011 - 01/2004)
6.	Priola, Suzette A: 7 articles (05/2009 - 09/2002)
7.	Singh, Neena: 6 articles (11/2015 - 12/2004)
8.	Langeveld, Jan P M: 6 articles (01/2014 - 07/2006)
9.	Ironside, James W: 6 articles (11/2010 - 01/2006)
10.	Kocisko, David A: 6 articles (03/2006 - 10/2003)

Related Diseases

1.	Scrapie 10/01/2009 - "To establish that PrP(res) infectivity was eliminated, we utilized the Rocky Mountain Laboratory (RML) mouse-adapted scrapie model system for bioassay. " 01/01/2014 - "In this study we examined 7 deviant ovine field cases of scrapie for some typical molecular aspects of PrP(res) found in CH1641‑scrapie, classical scrapie and BSE. " 03/01/2011 - "In the present study, using baculovirus-derived recombinant PrP (Bac-PrP), we demonstrated that Bac-PrP is useful as a PrP(C) substrate for amplification of the mouse scrapie prion strain Chandler, and PrP(Sc) that accumulated in mice inoculated with Bac-PrP(res) had biochemical and pathological properties very similar to those of the PrP(Sc) seed. " 01/01/2009 - "In a recent study, it was shown that sheep intracerebrally inoculated with a US scrapie agent (No. 13-7) developed scrapie and survived for an average of 19 months post inoculation. " 10/01/2004 - "The results from this study seem to confirm that the PrP(res) isolated from the BSE case in Alberta has similar molecular properties to the PrP(res) isolated from a BSE case in the UK, and that it differs in its molecular and immunological characteristics from the CWD and scrapie cases studied."
2.	Prion Diseases (Transmissible Spongiform Encephalopathies) 05/01/2004 - "The therapeutic efficacy of direct drug infusion into the brain, the target organ of transmissible spongiform encephalopathies, was assessed in transgenic mice intracerebrally infected with 263K scrapie agent. " 01/30/2006 - "Correlative studies support lipid peroxidation is linked to PrP(res) propagation as an early primary pathogenic event in prion disease." 11/29/2005 - "These studies provide new insight into the mechanism of prion polymerization, solve a long-standing practical problem in producing PrP-res fibrils from full-length PrP, and may help in identifying new genetic and environmental factors that modulate prion disease." 11/01/1999 - "Transmissible spongiform encephalopathies (TSE) or prion diseases result in aberrant metabolism of prion protein (PrP) and the accumulation of a protease-resistant, insoluble, and possibly infectious form of PrP, PrP-res. Studies of PrP biosynthesis, intracellular trafficking, and degradation has been studied in a variety of tissue culture cells. " 12/15/2015 - "Transmission of first-passage tg60CWD-H95(+) isolates into tg33 mice, however, elicited two prion disease presentations consistent with a mixture of strains associated with different PrP-res glycotypes. "
3.	Creutzfeldt-Jakob Syndrome (Creutzfeldt-Jakob Disease) 01/01/2009 - "To establish with improved accuracy the prevalence of disease related prion protein (PrP(CJD)) in the population of Britain and thereby guide a proportionate public health response to limit the threat of healthcare associated transmission of variant Creutzfeldt-Jakob disease (vCJD). " 07/26/2007 - "Molecular studies of PrP(res), followed by transmission studies to mice, gave the first evidence for the accidental transmission of the BSE agent to humans where it induced a variant form of the fatal Creutzfeldt-Jakob disease (CJD) and also to different animal species including a goat in France. " 06/01/2000 - "Immunohistochemical studies of the PrP(CJD) deposition in Creutzfeldt-Jakob disease." 08/07/2014 - "A test that detects the specific marker for Creutzfeldt-Jakob disease, the prion protein (PrP(CJD)), by means of real-time quaking-induced conversion (RT-QuIC) testing of cerebrospinal fluid has a sensitivity of 80 to 90% for the diagnosis of sporadic Creutzfeldt-Jakob disease. " 08/01/2009 - "Human variant Creutzfeldt-Jakob disease and sheep scrapie PrP(res) detection using seeded conversion of recombinant prion protein."
4.	Disease Susceptibility (Diathesis) 03/15/2007 - "We show that following inoculation with the scrapie agent by skin scarification, disease susceptibility was dramatically reduced in mice lacking the draining ILN. " 03/15/2007 - "Although early scrapie agent accumulation also occurs within the mesenteric lymph nodes, their presence in WT-->LTbeta(-/-) mice did not restore disease susceptibility. "
5.	Infection 10/01/2014 - "In the present study, we aimed to identify the potential roles of PAKs during prion infection, utilizing the brains of scrapie agent-infected hamsters. " 01/01/2004 - "Studies in rodents have shown that after peripheral infection, uptake of the scrapie agent is followed by an initial phase of replication in the lymphoreticular system, particularly the spleen and lymph nodes. " 05/01/2002 - "The aim of the study was to examine the role of gastric acidity in the protection of mice against infection after intragastric administration of different doses of a scrapie agent. " 09/01/2000 - "These studies show that the ileal Peyer's patch in young sheep may be an important site of uptake of the scrapie agent and that the biology of this major gut-associated lymphoid tissue may influence the susceptibility to oral infection in sheep. " 05/01/1997 - "In this paper we present a study of the spread of the scrapie agent after intraperitoneal infection of hamsters. "

Related Drugs and Biologics

1.	Amphotericin B (Amphotericin)
2.	MS 8209
3.	PrPC Proteins
4.	PrPSc Proteins
5.	Prions
6.	Haemophilus influenzae type b-polysaccharide vaccine-diphtheria toxoid conjugate
7.	Soil
8.	Stainless Steel (Steel, Stainless)
9.	Peptide Fragments
10.	Nucleic Acids

Related Therapies and Procedures

1.	Surgical Instruments (Clip)
2.	Therapeutics
3.	Splenectomy
4.	Intraperitoneal Injections
5.	Hyperbaric Oxygenation